A kick in the stomach..

As most of you know, a few weeks ago we went in for a CT scan of Parker's abdomen. He was sedated and his wonderful daddy was there waiting with bubbles when he woke up. Well, this week we got some news about this CT scan that felt like a kick in the stomach.

Let me start by giving you some back story. Parker's disease, ARPKD, comes with a liver disease known as CHF- Congenital Hepatic Fibrosis. If you have ARPKD, you automatically have CHF, because they come hand in hand. Most children don't show signs of CHF until later in life. Until now we have done routine tests and check ups with his GI doctor, but everything always seemed to be "fine". When the results of the CT  scan came back, though, they weren't. Parker's liver isn't draining right. When your liver doesn't drain right, it backs up and doesn't allow blood to flow through it. Parker's body, as amazing as it is, has been fighting to create a "natural shunt". He has created new veins and arteries by himself to bring blood around the liver and back to the heart. This is not good. It's called portal hypertension. 

The doctors here have met as a team, researched everything they could, and consulted other hospitals and doctors. This situation  isn't completely uncommon in patients with this disease. Parker is not the first child this has happened to, but his team has decided that this is out of their league. They don't have the experience to handle his transplant case. They spoke with several doctors and gave us several options as to who we should see. As of right now, we are waiting for a doctor from CHOP, Children's Hospital of Philadelphia to call us. They are the top children's hospital in the nation and have tons of experience in kidney/liver involvement. They are paired with the ARPKD/CHF Alliance and have a researcher specifically for CHF.

We don't know exactly what will happen. We don't know if he will need a liver transplant, or if they can use a renal-spleenal shunt to control things. We don't know how long we will be there, but have been told to expect to be there for months after transplant. I don't know if we are going there first for tests and then coming home or if we are going there and staying there. 

All that I do know, is that we have the most amazing donor. She has said that no matter what we are in this together and that she is willing to come with us to Philadelphia- over 1,000 miles. 

When I hear from the doctors at CHOP, I will update more. 

I should add that we have so much respect for our team. Their honesty with us is amazing. I applaud them for admitting that this is too much for them, instead of being egotistic. 

As you can imagine, we are having a hard time wrapping our heads around this. We know that our big guy is a fighter, but weren't expecting this obstacle for a while. Please keep us in your prayers. 

Lessons learned today...

1. It NEVER gets easier to leave my baby in sedation. No matter how big or small the procedure, I will cry.
2. Minutes feel like hours in that waiting room and my heart drops every time the door opens.
3. If I had trouble waiting one hour today, I will drive myself and everyone else crazy on day of transplant while waiting at least 7!
4. Nothing distracts you while in waiting. No social media, game, or book will help. You will read the same line 100 times.
5. If you have 100% battery life at 7 a.m., your phone will be dead by 3 p.m. I currently have 10% and its 2:20.
6. My husband is the best daddy, ever! Okay, I already knew this, but he reminded once more today. He bought Parker bubbles in the gift shop for when he woke up. Bubbles are Parker's favorite right now!
7. My child is absolutely hysterical when he is doped up! Of course I am biased, though.
8. Every doctors office and hospital is the slowest place on Earth. For a procedure that was supposed to happen at 10 a.m and only take an hour, we are just leaving. Once again, it's 2:30.

Before:












After:
















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Meeting the person who holds your child's life in their hands..

Today we met the transplant surgeon. When shaking the hand of the single person who will hold your child's life in their hands, you have mixed feelings. Do you get in their good graces, or threaten their life if they let anything happen to your baby? It's a tough decision. Today, our surgeon made that decision easy. We immediately felt comfortable with her.

Overall she is happy with where Parker is. We are going to do a few tests on his bladder, just to be safe. His bladder has never been used. Yes, I said never. She is consulting with Dr. Roth, who we love! Dr. Roth is the only surgeon who has ever operated on Parker. He performed Parker's nephrectomy and hydrocele repair, and Parker did really well after both of those. She will get his opinion in which tests to perform before the surgery and he may very well be in the operating room for the transplant as well. This makes us much more comfortable. We like familiar faces!

These bladder tests prior to transplant will tell us a lot. They will determine if he needs a bladder augmentation. They will determine if he will need to be catheterized for a few months after transplant. They will determine if he will need a drain after transplant. They will determine exactly how much his bladder can hold, and how much it will need to stretch.

She informed us that Children's uses a steroid free protocol. Instead, Parker will get three doses of strong immunosuppressants through a central line while we are inpatient. One of these doses will be immediately before the surgery. Each treatment will run over a course of about 6 hours. They chose this method because of the long term affects of steroids on children. I, for one, am happy about this. Not only for the long term reasons, but also because a child with "roid rage" after a major surgery doesn't sound pleasant.

She told us that since Parker is so young and extremely active-which she got to see first hand today- that she plans to keep him sedated for the first few days. I like this plan. Hopefully he will rest through the worst of the pain and recovering. I know that in the NICU, he tried pulling out every tube and wire possible, so I can only imagine that it will be much more difficult to control him now.

We talked about pain management and making sure that it is kept under control. It is a catch 22 with pain medicine, though. While it keeps Parker comfortable, it will also keep his bowels from waking up. This could lead to extreme constipation which is painful in itself. She agreed that he would need some sort of stool softener to make sure that he doesn't get "backed up". He won't be eating for the first few days, so hopefully this will help with things as well. She did say that she expected him to lose some weight. Since he is on dialysis, a lot of his weight is fluid related. When our new super kidney starts working it will be in over drive, so all of this fluid weight will disappear.

We asked her about the possibility of placing a g-tube during surgery. A g-tube is a feeding tube that is placed on his belly and goes directly into his stomach. This will be critical in the beginning to ensure that Parker can get enough fluid intake to keep his kidney happy, and to make sure he is getting his rejection medicine. When he is able to take the correct amount of fluid and all of his medicine by mouth for a little while, then we can discuss removing it. She was okay with the thought of this and said she would present it to the committee when they meet on Wednesday. I am eager to see my babies face without tubes and tape again!

We know that after transplant he will be on a lot of medicine, two of which will be extremely critical. These are his anti rejection medicines, Prograf and Cellcept. These medicines have to be given exactly 12 hours apart every day. The time frame is extremely crucial for these. He will also have labs 2-3 times a week for the first few months, as well as transplant clinic once a week. So the first few months will be extremely hectic.

Overall, we are at ease after our meeting today. We had a lot of questions answered today and are happy with the way things are going. She did mention that she has been doing all of the pediatric transplants. They have been doing 2-3 living donor transplants a week, so she's pretty experienced.


Here are a few new pictures of our big guy..












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